遗传性血管性水肿（HAE）中的补体、凝血和纤维蛋白溶解参数
Complement, coagulation and fibrinolytic parameters in hereditary angioedema (HAE).
作者信息
Cullmann W, Kövary P M, Müller N, Dick W
出版信息
Clin Exp Immunol. 1982 Sep;49(3):618-22.
Abstract
The intrinsic clotting, the kinin generating and the fibrinolytic systems were investigated in 10 patients with hereditary angioedema (HAE), 10 patients with chronic urticaria and 18 healthy volunteers. In spite of the fact that patients suffering from HAE severely lack C1 INH, neither the intrinsic coagulation nor the fibrinolytic systems are impaired. There was a slight decrease of plasma kallikrein--as already known--and moreover a greater decrease in HMW-kininogen, and increase in Factor XII levels. Furthermore, activation of pre-kallikrein was delayed in these patients. These findings make it apparent that lowered HMW-kininogen levels compensate the lack of C1 INH, thus preventing an enhanced activation of the intrinsic clotting and the fibrinolytic systems.
摘要
对10例遗传性血管性水肿（HAE）患者、10例慢性荨麻疹患者和18名健康志愿者的内源性凝血系统、激肽生成系统和纤维蛋白溶解系统进行了研究。尽管患有HAE的患者严重缺乏C1抑制物，但内源性凝血系统和纤维蛋白溶解系统均未受损。如已知的那样，血浆激肽释放酶略有下降，此外，高分子量激肽原下降幅度更大，而因子XII水平升高。此外，这些患者前激肽释放酶的激活延迟。这些发现表明，降低的高分子量激肽原水平补偿了C1抑制物的缺乏，从而防止内源性凝血系统和纤维蛋白溶解系统的过度激活。
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